G Chir Vol. 24 - n. 6/7 - pp. 243-245 Giugno-Luglio 2003
Communicating oesophageal duplication: a case report
M. DE FAZIO, A. GUGLIELMI, G. CATALANO
243
Introduction
Duplications of the oesophagus are rare
congeni-tal abnormalities of obscure origin which account for
10-15% of all duplications of the alimentary tract.
They usually manifest during the first year of life (5,
6, 10).
They may involve the thoracic oesophagus but can
extend below the diaphragm (3). Oesophageal
dupli-cations, either spherical or tubular, rarely
communi-cate with the oesophageal lumen, are usually lined by
gastric mucosa and have a smooth muscle coating.
They may lie centrally but more usually lie to one
side (3, 6, 11).
Duplications are commonly associated with other
congenital malformations, such as spinal deformities,
congenital heart disease, vertebral anomalies,
malrota-tion of the bowel, Meckel’s diverticulum (10).
A very rare case of a 26-year old man affected by
K l i p p e l - Trenaunay syndrome, with an asymptomatic
oesophageal duplication communicating prox i m a l l y
and distally, is reported.
Case report
A 26-year-old man, born with a port-wine hemangioma involving the neck, presented with dizziness and progressive weakness in the left extremities.
At physical examination, the left arm was longer than the right and showed clear hypertrophy of the soft tissue. There was moderate left hemiparesis, paresis of the soft palate with absence of the pha-ryngeal reflex, resulting in a high grade of neurological dysphagia.
A percutaneous endoscopic gastrostomy was performed. During this procedure, endoscopy of the upper gastrointestinal tract revealed division of the oesophagus into two lumens at 28 cm. with a narrow mucosal bridge 2 cm. above. It was possible to pass the endoscope through one lumen only for few cm. because of the progressive narrowing of the channel. The principal lumen communicated with the stomach through a normal oesopha-gogastric junction whereas the accessory lumen communicated distally with a small orifice just below that.
Histologic examination showed normal oesophageal epithelium. An upright contrast study of the oesophagus with Gastrografin showed a narrow tubular channel projecting from the left posterolat-eral aspect of the lower third of the oesophagus, slowly emptying into the stomach just below the oesophagogastric junction (Fig. 1). RI A S S U N T O: Duplicazione dell’esofago: descrizione di un caso
clinico.
M. DEFAZIO, A. GUGLIELMI, G. CATALANO
Le duplicazioni esofagee sono rare anomalie congenite che spesso non comunicano con il lume esofageo. Sono comunemente associate con altre malformazioni congenite, come deformità spinali, malattie cardiache congenite, anomalie vertebrali, malrotazioni intestinali, diverticolo di Meckel.
Durante l’esecuzione di una gastrostomia endoscopica percutanea per una disfagia neurologica, l’endoscopia metteva in evidenza un rarissimo caso di sindrome di Klippel-Trenaunay in un giovane uomo di 26 anni, con una asintomatica duplicazione esofagea comunicante sia prossimamente che distalmente.
KE YWO R D S: Oesophageal duplication - Klippel-Trenaunay syndrome - Percutaneous endoscopic gastrostomy.
Duplicazione dell’esofago - Sindrome di Klippel-Trenaunay - Gastrostomia endoscopica percutanea .
University of Bari, Italy
Department of Emergency and Organ Transplantation General Surgery and Liver Transplantation (Chief: Prof. V. Memeo)
© Copyright 2003, CIC Edizioni Internazionali, Roma
Abbreviations: Klippel-Trenaunay syndrome (KTS), percutaneous endoscopic gastro-stomy (PEG).
SU M M A R Y: Communicating oesophageal duplication: a case
report.
M. DEFAZIO, A. GUGLIELMI, G. CATALANO
Duplications of the oesophagus are rare congenital abnormalities and rarely comunicate with the oesophageal lumen. They are com -monly associated with other congenital malformations, such as spinal deformities, congenital heart disease, vertebral anomalies, malrota -tion of the bowel, Meckel’s diverticulum.
During a percutaneous endoscopic gastrostomy, performed becau -se of a neurological dysphagia, the endoscopy revealed a very rare ca-se of a 26-year old man affected by Klippel-Trenaunay syndrome, with an asymptomatic oesophageal duplication that communicated proxi -mally and distally.
The patient was submitted to magnetic resonance imaging and angiography that revealed a fusiform aneurysm of the right vertebral artery, some aneurysmatic dilatation of the distal tract of the left vertebral artery and a giant sack-like basilar artery aneurysm (Fig. 2).
To reduce the strong compression on the brain stem, occlu-sion of both the vertebral arteries was accomplished in two steps. The patient tolerated both treatments and the neurological signs remained unchanged.
He subsequently followed a rehabilitation program.
Discussion
The criteria suggested by Ladd and Gross in 1944
for diagnosis of duplication are: adherence to some
part of the alimentary tract; the presence of a smooth
muscle wall, usually in two layers; and an internal
lin-ing correspondlin-ing to some part of the alimentary
tract (10). The particular features of this case are the
absence of symptoms due to the double
communica-tion, proximal and distal, of the accessory lumen with
the alimentary tract, the normal lining with
epider-moid epithelium and the association with multiple
vascular abnormalities typical of Klipper-Tre n a u n a y
syndrome (KTS) (7-9).
In this syndrome, venous varicosities can also
i n vo l ve intra-abdominal and intra-pelvic organs:
g a s t rointestinal bleedings, basically from colonic
vascular malformations or oesophageal va r i c e s
caused by hypoplasia of the portal vein, are also
re p o rted (2).
The association of arterial and venous anomalies
could be explained by the hypothesis of a congenital
d i s o rder resulting from a mesodermal abnormality
occuring during fetal development (1). KTS had been
suggested to result from the action of a mosaic gene
abnormality that is lethal to the gamete when present
in all cells of the embryo (4).
M. De Fazio e Coll.
244
Fig. 1 - Oesophageal X-ray after ingestion of Gastrografin. In the third distal is evident the lumer duplication.
References
1. Baskerville P, Ackroyd J Browse N: The etiology of Klippel-Trenaunay syndrome. Ann Surg 202: 624, 1985.
2. Bataller R, Sans M, Escorsell A, Elizalde JI, Bosch J, Rodes J: Esophageal variceal bleeding caused by hypoplasia of the por-tal vein in a patient with the Klippel-Trenaunay syndrome. Am J Gastroenterol 1998, 93: 274.
3. Grosfeld Jay L, James A, O’Neill JR, Clatworthy HW: Enteric duplications in infancy and childhood: an 18-year review. Ann Surg 1970; 172: 83.
4. Happle R: Lethal genes surviving by mosaicism: a possible explanation for sporadic birth defects involving the skin. I Am Acad Dermatol 1987; 16: 899.
5. Karahasanoglu T, Ozbal A, Alcicek S, Goksel S, Altun M: Giant intra-abdominal esophageal duplication cyst. Endosco-py 1997; 29: 554.
6. Kirkland Ruffin W and Dale Hansen E:An esophageal dupli-cation cyst presenting as an abdominal mass. Am J Gastro-enterol 1989; 84: 571.
7. Nakamura K, Onitsuka T, Koga Y, Shibata K, Tsuchida Y, Nabeshima K: Aneurysm of the transverse cervical artery occurring in association with a cavernous hemangioma as a complication of Klippel-Trenaunay syndrome: report of a case. Surg Today 1995; 25: 978.
8. Paes EHJ, Vollmar JF: Aneurysma transformation in conge-nital venous angiodysplasias in lower extremities. Int Angiol 1990; 9: 90.
9. Phillips GN, Gordon DH, Martin EC, Haller JO, Casarella W: The Klippel-Trenaunay syndrome: clinical and radiologi-cal aspects. Radiology 1978; 128: 429.
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This abnormality can also interfere with the
devel-opment of the oesophagus, either pre venting the
re g ression of embryological dive rticula (according to
the dive rticulum theory of Lewis and Thyng) or, in
the stage of vacuolation, favouring the isolation of
some of these vacuoles that coalesce to form a parallel
tube (according to Sir Arthur Keith’s vacuolation
the-ory) (10). The variety of clinical features is probably
due to the stage of embryogenesis when the nox a e
operate.
Communicating oesophageal duplication: a case report
